Poorer Adaptive Ability Tied to Anxiety and Hyperactivity in PWS, Study Says

Poorer Adaptive Ability Tied to Anxiety and Hyperactivity in PWS, Study Says

Difficulties that people with Prader-Willi syndrome (PWS) have in adapting to their surroundings appear to be linked to psychiatric problems, generalized anxiety and hyperactivity, researchers report.

Their study, “The Profiles and Correlates of Psychopathology in Adolescents and Adults with Williams, Fragile X and Prader-Willi Syndromes,” which also looked at people with two similar disorders, was published in the Journal of Autism and Developmental Disorders.

Psychopathology is the field of science focused on mental disorders, including their causes, development, classification, and treatment.

The prevalence of mental illnesses that fall within the scope of psychopathology is estimated to be four-to-five times higher among individuals with some form of intellectual disability — including PWS, Williams syndrome and fragile X syndrome — than in the general population.

Although the degrees of intellectual impairment is similar in people with one of these three syndromes, significant differences exist as to the nature of their mental disorders. Studies have shown a high prevalence of psychotic illness in PWS patients, social anxiety in those with fragile X, and generalized anxiety and phobias (irrational fears) in individuals with Williams syndrome.

Little is known about what causes these differences, and the specific mental health aspects that distinguish these syndromes.

Researchers in the U.K. asked caregivers of people with PWS, fragile X, and Williams syndrome to complete questionnaires regarding the impact of these disorders on the ability the person they are caring for to engage in normal life activities, their overall health, and their levels of anxiety and depression.

They then used statistical analyses to identify potential relationships between four possible predictors of mental illness — age, adaptive ability, health problems, and sensory impairments — and specific features of each of the three syndromes. (Adaptive ability refers to a capacity to adapt to surroundings and avoid engaging in disrupting behaviors.)

A total of 111 caregivers (mean age, 26) of patients ages 12 and old completed the surveys. Among them, 26 were caring for  people with PWS, 35 for those with WS, and 50 for people with fragile X.

Results of the analysis linked the poorer adaptive ability evident in PWS patients to greater psychiatric disturbances, generalized anxiety, and hyperactivity or manic behavior.

For those with Williams syndrome, poor overall health (high rates of health issues) and problems with the five senses (sensory impairments) were associated with more emotional and behavioral problems, and with social avoidance.

No associations were found among those with fragile X .

“We identified significant and potentially important cross-syndrome differences in the correlates of psychopathology for individuals with [Williams syndrome], PWS and [fragile X],” the investigators wrote.

“This study highlights the need for continued research into risk markers, utilizing a combination of approaches and the development of targeted syndrome-specific interventions to reduce and manage psychopathological disorders,” they added.

Joana is currently completing her PhD in Biomedicine and Clinical Research at Universidade de Lisboa. She also holds a BSc in Biology and an MSc in Evolutionary and Developmental Biology from Universidade de Lisboa. Her work has been focused on the impact of non-canonical Wnt signaling in the collective behavior of endothelial cells — cells that make up the lining of blood vessels — found in the umbilical cord of newborns.
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José is a science news writer with a PhD in Neuroscience from Universidade of Porto, in Portugal. He has also studied Biochemistry at Universidade do Porto and was a postdoctoral associate at Weill Cornell Medicine, in New York, and at The University of Western Ontario in London, Ontario, Canada. His work has ranged from the association of central cardiovascular and pain control to the neurobiological basis of hypertension, and the molecular pathways driving Alzheimer’s disease.
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Joana is currently completing her PhD in Biomedicine and Clinical Research at Universidade de Lisboa. She also holds a BSc in Biology and an MSc in Evolutionary and Developmental Biology from Universidade de Lisboa. Her work has been focused on the impact of non-canonical Wnt signaling in the collective behavior of endothelial cells — cells that make up the lining of blood vessels — found in the umbilical cord of newborns.
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